Clinical risk stratification of children with SIOPEL high-risk hepatoblastoma in Taiwan

• Main Authors: Wei-Yun Hsu, Hsiu-Hao Chang, Meng-Yao Lu, Yung-Li Yang, Shiann-Tarng Jou, Huey-Ling Chen, Yen-Hsuan Ni, Hong-Yuan Hsu, Mei-Hwei Chang, Jia-Feng Wu
• Format: Article
• Language: English
• Published: Elsevier 2020-08-01
• Series: Pediatrics and Neonatology
• Subjects: Hepatoblastoma; Risk stratification; SIOPEL
• Online Access: http://www.sciencedirect.com/science/article/pii/S1875957220300528

Backgorund: Hepatoblastoma is the most common primary liver malignancy in young children. Methods: To identify predictors of the clinical outcomes of hepatoblastoma, we retrospectively reviewed the medical records of 45 children with hepatoblastoma in the National Taiwan University Hospital from 1998 to 2018. All of the children were classified as high risk according to the pretreatment extent of disease (PRETEXT) staging system. The patients’ clinical data (sex, age at diagnosis, PRETEXT status, presence of metastasis or tumor rupture, tumor pathologic type, and clinical outcomes) were analyzed. Results: A total of 45 children with high-risk hepatoblastoma were diagnosed at an average age of 3.2 years. The survival analysis showed that the event-free survival duration was significantly longer in patients aged ≤1.25 years at diagnosis than those >1.25 years (hazard ratio = 2.86, p = 0.036). The absence of initial tumor rupture was associated with longer event-free survival (hazard ratio = 2.74, p = 0.039). Diagnosis at age >1.25 years was correlated with the presence of multifocal liver tumors (p = 0.0002) and tumor rupture at diagnosis (p = 0.02). There was no significant difference in event-free survival between the groups classified as intermediate versus high risk according to the Children's Hepatic tumors International Collaboration hepatoblastoma stratification system (p = 0.13). Conclusions: Diagnosis at ≤ 1.25 years of age and absence of initial tumor rupture were predictive of a good clinical prognosis in Taiwanese children with hepatoblastoma.